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Quantification of Age-Dependent Somatic CAG Repeat Instability in Hdh CAG Knock-In Mice Reveals Different Expansion Dynamics in Striatum and Liver

BACKGROUND: Age at onset of Huntington's disease (HD) is largely determined by the CAG trinucleotide repeat length in the HTT gene. Importantly, the CAG repeat undergoes tissue-specific somatic instability, prevalent in brain regions that are disease targets, suggesting a potential role for som...

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Autores principales: Lee, Jong-Min, Pinto, Ricardo Mouro, Gillis, Tammy, St. Claire, Jason C., Wheeler, Vanessa C.
Formato: Artigo
Lenguaje:Inglês
Publicado: Public Library of Science 2011
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3163641/
https://ncbi.nlm.nih.gov/pubmed/21897851
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0023647
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